Digestive involvement in Primary Sjogren Syndrome

Abstract

Sjo¨gren's syndrome (SS) is a systemic autoimmune disease that mainly affectsthe exocrine glands and usually presents as persistent dryness of the mouth andeyes due to functional impairment of the salivary and lacrimal glands. The histological hallmark is a focal lymphocytic infiltration of the exocrine glands, and the spectrum of the disease extends from an organ-specific autoimmune disease (autoimmune exocrinopathy)to a systemic process with diverseextraglandular manifestations. An estimated 2e4 million people in the United States have SS, and approximately 1 million of them have an established diagnosis. The prevalence in European countries ranges between 0.60 and 3.3%. The incidence of SS has been calculated as 4 cases per 100,000. SS primarilyaffects Caucasian perimenopausal women, with a femaleemale ratio rangingfrom 14:1 to 24:1 in the largest reported series. The disease may occur at all ages but typically has its onset in the fourthesixth decades of life; although some cases are detected in younger female patients, especially in mothers of babies with congenital heart block. When sicca symptoms appear in a previously healthy person, the syndrome is classified as primary SS. When sicca features are found in association with another systemic autoimmune disease, most commonly rheumatoid arthritis (RA), systemic sclerosis (SSc), or systemic lupus erythematosus (SLE), it is classified as associated SS. The variability in the presentation of SS may partially explain the delays in diagnosis of up to 9 years from the onset of the symptoms. Although most patients present with sicca symptoms, various clinical and analytical features may indicate an undiagnosed SS. In addition, SS is a disease that may be expressed in many guises, depending on the specific epidemiological, clinical, or immunologic features. Epidemiologically, a lower frequency of autoantibodies is observed in male SS patients and those with an earlier onset.Clinically, two main patterns of disease expression are observed: patients withonly glandular involvement (sicca-limited disease), who have a low frequencyof immunologic abnormalities and extraglandular features; and patients with apredominant systemic expression in addition to the sicca involvement.Patients with positive immunologic features need a closer follow-up, payingspecial attention to the development of extraglandular manifestations. Thetherapeutic management of SS is mainly centered on the control of siccafeatures, using substitutive and oral muscarinic agents, while corticosteroidsand immunosuppressive agents play a key role in the treatment of extraglandular features.Gastrointestinal involvement has been little studied in primary SS and mayinclude altered esophageal motility, gastroesophageal reflux (GER), chronicgastritis, and, less frequently, malabsorption. More clinical data are availableon pancreatic and liver involvements, which were first reported to be part ofthe extraglandular expression of SS by Bloch et al.